HUS
MCID: HML001
MIFTS: 54

Hemolytic-Uremic Syndrome (HUS)

Categories: Blood diseases, Immune diseases, Nephrological diseases, Rare diseases

Aliases & Classifications for Hemolytic-Uremic Syndrome

MalaCards integrated aliases for Hemolytic-Uremic Syndrome:

Name: Hemolytic-Uremic Syndrome 12 75 29 55 6 44 15 72
Hemolytic Uremic Syndrome 12 53 59
Haemolytic-Uraemic Syndrome 12 33
Hus 53 59
Acute Renal Failure, Thrombocytopenia, and Microangiopathic Hemolytic Anemia Associated with Distorted Erythrocytes 53

Classifications:



External Ids:

Disease Ontology 12 DOID:12554
ICD9CM 35 283.11
MeSH 44 D006463
NCIt 50 C75545
ICD10 33 D59.3
Orphanet 59 ORPHA544458
UMLS 72 C0019061

Summaries for Hemolytic-Uremic Syndrome

NIH Rare Diseases : 53 Hemolytic uremic syndrome (HUS) is a disorder that usually occurs when an E. coli bacterial infection in the digestive system produces toxic substances that destroy red blood cells. Symptoms include vomiting and diarrhea, fever, lethargy, and weakness. In severe cases it can lead to kidney failure or death. While this condition is most common in children, it often has a more complicated presentation in adults. Treatment may include dialysis, corticosteroids, transfusions of packed red blood cells and plasmapheresis. Hemolytic uremic syndrome should be distinguished from atypical hemolytic uremic syndrome (aHUS). The two conditions have different causes and different signs and symptoms.

MalaCards based summary : Hemolytic-Uremic Syndrome, also known as hemolytic uremic syndrome, is related to nephrotic syndrome, type 7 and complement factor h deficiency. An important gene associated with Hemolytic-Uremic Syndrome is DGKE (Diacylglycerol Kinase Epsilon), and among its related pathways/superpathways are Innate Immune System and Creation of C4 and C2 activators. The drugs Abatacept and Prednisolone phosphate have been mentioned in the context of this disorder. Affiliated tissues include kidney, endothelial and bone, and related phenotypes are hematopoietic system and cardiovascular system

Disease Ontology : 12 A kidney disease that is characterized by hemolytic anemia, thrombocytopenia, and renal failure caused by platelet thrombi in the microcirculation of the kidney and other organs.

Wikipedia : 75 Hemolytic-uremic syndrome (HUS) is a group of blood disorders characterized by low red blood cells,... more...

Related Diseases for Hemolytic-Uremic Syndrome

Diseases in the Hemolytic-Uremic Syndrome family:

D-Plus Hemolytic Uremic Syndrome

Diseases related to Hemolytic-Uremic Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 787)
# Related Disease Score Top Affiliating Genes
1 nephrotic syndrome, type 7 34.7 DGKE CFH
2 complement factor h deficiency 33.3 CFI CFHR1 CFH CD46
3 proliferative glomerulonephritis 31.7 CFH C3
4 hemolytic anemia 31.4 HP CFI CFH C3 ADAMTS13
5 complement deficiency 31.1 CFH C3
6 neisseria meningitidis infection 31.1 CD46 C3
7 hemolytic uremic syndrome, atypical 1 31.1 VWF THBD DGKE CFI CFHR5 CFHR3
8 complement factor i deficiency 31.0 CFI C3
9 purpura 30.8 VWF THBD F3 ADAMTS13
10 thrombotic thrombocytopenic purpura, congenital 30.8 HP CFH ADAMTS13
11 disseminated intravascular coagulation 30.7 THBD F3 ADAMTS13
12 raynaud phenomenon 30.7 VWF THBD
13 membranoproliferative glomerulonephritis 30.6 CFHR5 CFH CFB CD46 C3
14 c3 glomerulopathy 30.5 CFHR5 CFHR3 CFHR1 CFB C3
15 evans' syndrome 30.5 HP ADAMTS13
16 glomerulonephritis 30.2 CFI CFHR5 CFH CD46 CD40LG C3
17 dense deposit disease 30.1 DGKE CFHR5 CFH CFB C3
18 anemia, autoimmune hemolytic 30.0 HP CD40LG C3
19 thrombotic thrombocytopenic purpura 30.0 VWF THBD F3 CFH CD46 ADAMTS13
20 vasculitis 30.0 VWF THBD CFI CD40LG
21 thrombophilia 30.0 VWF THBD F3
22 immunoglobulin alpha deficiency 30.0 HP CD40LG
23 hemophilia 29.9 VWF F3
24 spotted fever 29.9 VWF THBD
25 intermediate coronary syndrome 29.6 VWF F3
26 primary thrombocytopenia 29.5 VWF CD40LG ADAMTS13
27 korean hemorrhagic fever 29.5 VWF F3
28 enterocolitis 29.4 CFI CFHR5 CFHR3 CFHR1 CFH CFB
29 thrombophilia due to thrombin defect 29.4 VWF THBD F3
30 peripheral vascular disease 29.4 VWF THBD F3
31 heparin-induced thrombocytopenia 29.4 F3 CD40LG
32 von willebrand's disease 29.3 VWF F3 ADAMTS13
33 macular degeneration, age-related, 1 29.3 CFI CFHR5 CFHR3 CFHR1 CFH CFB
34 hemophilia a 29.2 VWF F3
35 thrombosis 29.2 VWF THBD F3 ADAMTS13
36 vascular disease 29.1 VWF THBD HP F3
37 afibrinogenemia 29.1 VWF F3 CFI CD46 C3
38 pulmonary hypertension 29.0 VWF THBD F3
39 catastrophic antiphospholipid syndrome 29.0 F3 CFH CD40LG ADAMTS13
40 essential thrombocythemia 28.9 VWF THBD F3
41 antiphospholipid syndrome 28.5 VWF THBD F3 CD40LG ADAMTS13
42 systemic lupus erythematosus 28.0 VWF THBD F3 CFB CD40LG C3
43 myocardial infarction 28.0 VWF THBD F3 CD40LG ADAMTS13
44 hellp syndrome 28.0 VWF THBD HP F3 CFI CFH
45 malaria 27.8 VWF THBD HP F3 CD40LG ADAMTS13
46 hemolytic uremic syndrome, atypical 3 13.0
47 hemolytic uremic syndrome, atypical 4 13.0
48 hemolytic uremic syndrome, atypical 5 13.0
49 hemolytic uremic syndrome, atypical 6 13.0
50 hemolytic uremic syndrome, atypical 2 13.0

Comorbidity relations with Hemolytic-Uremic Syndrome via Phenotypic Disease Network (PDN):


Acute Kidney Failure Deficiency Anemia
Heart Disease

Graphical network of the top 20 diseases related to Hemolytic-Uremic Syndrome:



Diseases related to Hemolytic-Uremic Syndrome

Symptoms & Phenotypes for Hemolytic-Uremic Syndrome

MGI Mouse Phenotypes related to Hemolytic-Uremic Syndrome:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.96 ADAMTS13 C3 CD40LG CFB CFH CFI
2 cardiovascular system MP:0005385 9.92 C3 CD40LG CD46 CFH F3 HP
3 homeostasis/metabolism MP:0005376 9.85 ADAMTS13 C3 CD40LG CFB CFH CFI
4 immune system MP:0005387 9.61 ADAMTS13 C3 CD40LG CFB CFH F3
5 renal/urinary system MP:0005367 9.17 C3 CD40LG CFB CFH CFI HP

Drugs & Therapeutics for Hemolytic-Uremic Syndrome

Drugs for Hemolytic-Uremic Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 90)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Abatacept Approved Phase 4 332348-12-6 10237
2
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
3
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
4
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
5
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
6
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
7 Hematinics Phase 4
8 Epoetin alfa Phase 4 113427-24-0
9 Prednisolone acetate Phase 4
10 Methylprednisolone Acetate Phase 4
11
Ravulizumab Approved, Investigational Phase 3 1803171-55-2
12 Complement Inactivating Agents Phase 3
13 Shiga Toxins Phase 3
14
Acetaminophen Approved Phase 2 103-90-2 1983
15
Promethazine Approved, Investigational Phase 2 60-87-7 4927
16
Diphenhydramine Approved, Investigational Phase 2 58-73-1, 147-24-0 3100
17
Triamcinolone Approved, Vet_approved Phase 2 124-94-7 31307
18
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
19
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3
20 Immunosuppressive Agents Phase 2
21 triamcinolone acetonide Phase 2
22 Triamcinolone diacetate Phase 2
23 Triamcinolone hexacetonide Phase 2
24 Liver Extracts Phase 2
25 Immunologic Factors Phase 2
26 Complement System Proteins Phase 2
27 Hormones Phase 2
28 Androgens Phase 2
29 Hormone Antagonists Phase 2
30 Androgen Antagonists Phase 2
31 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2
32 Androgen Receptor Antagonists Phase 2
33 Immunotoxin HA22 Phase 2
34 Immunotoxins Phase 2
35 BB 1101 Phase 2
36
Aspirin Approved, Vet_approved Phase 1 50-78-2 2244
37
rituximab Approved Phase 1 174722-31-7 10201696
38 Angiotensin-Converting Enzyme Inhibitors Phase 1
39 Hydroxymethylglutaryl-CoA Reductase Inhibitors Phase 1
40 Vaccines Phase 1
41 Antineoplastic Agents, Immunological Phase 1
42 Antirheumatic Agents Phase 1
43 Antibodies Phase 1
44 Antibodies, Monoclonal Phase 1
45 Immunoglobulins Phase 1
46
Metoclopramide Approved, Investigational 364-62-5 4168
47
Methotrexate Approved 1959-05-2, 59-05-2 126941
48
leucovorin Approved 58-05-9 143 6006
49
Azithromycin Approved 83905-01-5 447043 55185
50
Prednisone Approved, Vet_approved 53-03-2 5865

Interventional clinical trials:

(show top 50) (show all 61)
# Name Status NCT ID Phase Drugs
1 Effect of Erythropoietin on Red Blood Cell Requirement in Children With Hemolytic Uremic Syndrome: a Randomized Controlled Trial Recruiting NCT03776851 Phase 4 erythropoietin
2 Randomized Conversion Of Epstein-Barr Virus (EBV)+ Kidney Transplant Recipients Of Living Or Standard Criteria Donors At Three Months Post Transplantation To Belatacept With MPA Or Belatacept With Low-Dose Tacrolimus (50% Of Dose) Compared To Patients Remaining On Center Specific Standard Therapy Of Tacrolimus And MPA Recruiting NCT02213068 Phase 4 belatacept;Tacrolimus;MPA
3 Evaluation of Potential Predictors of Disease Progression in Patients With aHUS, Including Genetics, Biomarkers and Treatment Active, not recruiting NCT02614898 Phase 4
4 Multicentric, Prospective Open-label Study Assessing an Algorithm-based Strategy of Eculizumab Discontinuation in Children and Adults With Atypical Hemolytic Uremic Syndrome (aHUS) Active, not recruiting NCT02574403 Phase 4 eculizumab
5 An Open-Label, Multi-Center Trial of Eculizumab in Patients With Shiga-Toxin Producing Escherichia Coli Hemolytic-Uremic Syndrome (STEC-HUS) Completed NCT01410916 Phase 2, Phase 3 Eculizumab (Soliris®)
6 Early Treatment With the Monoclonal C5 Antibody Eculizumab in Pediatric Patients Affected by Shiga-toxin Related Hemolytic and Uremic Syndrome: A Phase III Prospective Randomized Controlled Therapeutic Trial Versus Placebo Completed NCT02205541 Phase 3 Eculizumab;Placebo
7 A Phase 3, Open-Label, Multicenter Study of ALXN1210 in Children and Adolescents With Atypical Hemolytic Uremic Syndrome (aHUS) Recruiting NCT03131219 Phase 3
8 A Phase 3 Study to Evaluate the Safety and Efficacy of OMS721 for the Treatment of Atypical Hemolytic Uremic Syndrome (aHUS) in Adults and Adolescents Recruiting NCT03205995 Phase 3
9 CONSERVE: rVA576 (Coversin) Long Term Safety and Efficacy Surveillance Study Recruiting NCT03829449 Phase 3 rVA576 (Coversin)
10 Improvements Through the Use of a Rapid Multiplex PCR Enteric Pathogen Detection Kit in Children With Hematochezia Recruiting NCT03362970 Phase 3
11 Single Arm Study of ALXN1210 in Complement Inhibitor Treatment-Naïve Adult and Adolescent Patients With Atypical Hemolytic Uremic Syndrome (aHUS) Active, not recruiting NCT02949128 Phase 3
12 Phase III Randomized Study of SYNSORB Pk in Children With E. Coli-Associated Terminated NCT00004465 Phase 3 SYNSORB Pk;Placebo
13 The Plasma Large-Volume Exchange Randomized Controlled Trial (PLEX-RCT) Withdrawn NCT01433003 Phase 3
14 A Phase II Study Evaluating the Efficacy of Rituximab in the Management of Patients With Relapsed/Refractory Thrombotic Thrombocytopenic Purpura (TTP) - Hemolytic Uremic Syndrome (HUS) Unknown status NCT00531089 Phase 2 Rituximab
15 The Safety and Efficacy of Eculizumab in Japanese Patients With Atypical Hemolytic Uremic Syndrome (aHUS) Completed NCT01757431 Phase 2 Eculizumab
16 An Open-Label, Multi-Center Clinical Trial of Eculizumab in Pediatric Patients With Atypical Hemolytic-Uremic Syndrome Completed NCT01193348 Phase 2 Eculizumab
17 An Open-label, Multi-center Clinical Trial of Eculizumab in Adult Patients With Atypical Hemolytic-uremic Syndrome Completed NCT01194973 Phase 2 Eculizumab
18 An Open-Label, Multi-Center Controlled Clinical Trial of Eculizumab in Adolescent Patients With Plasma Therapy-Resistant Atypical Hemolytic Uremic Syndrome (aHUS) Completed NCT00844844 Phase 2 Eculizumab
19 An Open-Label, Multi-Center Controlled Clinical Trial of Eculizumab in Adult Patients With Plasma Therapy-Resistant Atypical Hemolytic Uremic Syndrome (aHUS) Completed NCT00844545 Phase 2 Eculizumab
20 An Open-Label, Multi-Center Controlled Clinical Trial Of Eculizumab in Adolescent Patients With Plasma Therapy-Sensitive Atypical Hemolytic Uremic Syndrome (AHUS) Completed NCT00844428 Phase 2 eculizumab
21 An Open-label, Multi-center Controlled Clinical Trial of Eculizumab in Adult Patients With Plasma Therapy-sensitive Atypical Hemolytic Uremic Syndrome (AHUS) Completed NCT00838513 Phase 2 eculizumab
22 A Phase II Study of Chimeric Monoclonal Antibodies to Shiga Toxins 1 (cαStx1) and 2 (cαStx2) Administered Concomitantly to Children With Shiga Toxin-Producing Bacterial (STPB) Infection and Bloody Diarrhea (SHIGATEC Trial) Completed NCT01252199 Phase 2 cαStx1/cαStx2;Placebo
23 Early Intervention With Eculizumab to Treat Thrombotic Microangiopathy/Atypical Hemolytic Uremic Syndrome (TMA/aHUS)-Associated Multiple Organ Dysfunction Syndrome (MODS) in Hematopoietic Stem Cell Transplant (HCT) Recipients Recruiting NCT03518203 Phase 2 Eculizumab
24 A Phase 2, Uncontrolled, Three-Stage, Dose-Escalation Cohort Study to Evaluate the Safety, Pharmacokinetics, Pharmacodynamics, Immunogenicity, and Clinical Activity of OMS721 in Adults With Thrombotic Microangiopathies Recruiting NCT02222545 Phase 2
25 A Phase II Clinical Trial of Anti-Tac(Fv)-PE38 (LMB-2) Immunotoxin for CD25 Positive Hairy Cell Leukemia Active, not recruiting NCT00321555 Phase 2 Anti-Tac(Fv)-PE38 (LMB-2) Immunotoxin
26 A Phase II, Randomized, Double-blind, Placebo-controlled Trial to Evaluate the Safety and Efficacy of Cemdisiran (ALN-CC5) Following Withdrawal of Chronic Eculizumab Therapy in Patients With Atypical HUS at High Risk of Recurrence Not yet recruiting NCT03999840 Phase 2 cemdisiran;Placebos
27 An Open-label Phase 2 Study to Assess the Effect of C5aR Antagonist Therapy by CCX168 Oral Administration on ex Vivo Thrombus Formation and Disease Activity in ESRD Patients With Atypical Hemolytic Uremic Syndrome Terminated NCT02464891 Phase 2 CCX168
28 A Phase II Study of the Anti-CD22 Recombinant Immunotoxin Moxetumomab Pasudotox (CAT-8015, HA22) in Children With B-lineage Acute Lymphoblastic Leukemia and Minimal Residual Disease Prior to Allogeneic Hematopoietic Stem Cell Transplantation Terminated NCT02338050 Phase 2
29 Retreatment Protocol for BL22 Immunotherapy in Relapsed or Refractory Hairy Cell Leukemia Terminated NCT00924040 Phase 2 BL22 (CAT-3888)
30 A Phase 2, Open Label, Multicenter Study of ALN-CC5 Administered Subcutaneously in Adult Patients With Atypical Hemolytic Uremic Syndrome Withdrawn NCT03303313 Phase 2 Cemdisiran
31 A Phase I Study to Evaluate the Safety, Tolerance and Pharmacokinetics of the Anti-Shiga Toxin Hyperimmune Equine Immunoglobulin F(ab')2 Fragment (INM004) in Healthy Volunteers Completed NCT03388216 Phase 1
32 Eculizumab Therapy for Dense Deposit Disease and C3 Nephropathy Completed NCT01221181 Phase 1 Eculizumab
33 Inpatient Volume Expansion in Children With Shiga Toxin-Producing Escherichia Coli (STEC) Infection to Prevent Hemolytic Uremic Syndrome (HUS) Not yet recruiting NCT03275792 Phase 1 D5-0.9%NS;Routine home oral rehydration
34 A Phase I Study of Moxetumomab Pasudotox-tdfk (Lumoxiti) and Rituximab (Rituxan (R)) for Relapsed Hairy Cell Leukemia Not yet recruiting NCT03805932 Phase 1 Moxetumomab Pasudotox-tdfk
35 Complement Activation During Hemodialysis in Atypical Hemolytic Uraemic Syndrome as Underlying Kidney Disease. Unknown status NCT00930423
36 Study of 'Vascular Competence' Profile and Endothelial Activation in the Hemolytic Uremic Syndrome in Children and Adults Unknown status NCT02904863
37 A Retrospective, Observational, Non-interventional Trial to Assess Eculizumab Treatment Effect in Patients With Atypical Hemolytic Uremic Syndrome (aHUS) Completed NCT01770951
38 A Research Study to Describe the Safety and Efficacy of Eculizumab in Japanese Patients With Atypical Hemolytic Uremic Syndrome (aHUS). An Assessment of Two Case Studies Completed NCT01755429
39 Haemolytic Uraemic Syndrome in Childhood: Clinical, Cognitive and Psychological Aspects Completed NCT01666548
40 Outbreak of Hemolytic Uremic Syndrome Linked to Escherichia Coli of Serotype O104:H4 in Bordeaux Urban Area, June 2011: Evaluation of Diagnostic, Prognostic and Pathophysiological Data Completed NCT01406288
41 Repetitive Intestinal Lavage Using Polyethylene Glycol Solution in Patients With EHEC O104:H4 Infection During the German 2011 Outbreak for Prevention of Severe Thrombocytopenia With Subsequently Following Therapeutic Plasmapheresis Completed NCT01561248 polyethylene glycol solution for daily bowel lavage.
42 The Role of Endothelium Dysfunction in Progression of CKD (Chronic Kidney Disease) After AKI (Acute Kidney Injury) Completed NCT00358306
43 Association Between the Quantitative Assessment of Schistocytes in Peripheral Blood Smear and Prognosis of Patient Initially Diagnosed as HELLP Syndrome. Completed NCT03246542
44 Assessment of Immature Platelet Fraction in Pregnancy-Associated Thrombotic Microangiopathy Completed NCT03232359
45 Safety of Tacrolimus And Methotrexate (MTX) Versus Tacrolimus And Mycophenolate Mofetil (MMF) As Graft Versus Host Disease Prophylaxis In Allogeneic Hematopoietic Cell Transplants (HCT) Completed NCT00360685 Tac+MTX;TAC + MMF
46 A Observational Study to Determine the Prevalence of Pregnancy-related Thrombotic Thrombocytopenic Purpura and Atypical Haemolytic Uraemic Syndrome in Women Affected by Specific Obstetric Complications Recruiting NCT03605511
47 An Observational, Non-Interventional, Multi-Center, Multi-National Study of Patients With Atypical Hemolytic-Uremic Syndrome (aHUS Registry) Recruiting NCT01522183
48 Coordination of Rare Diseases at Sanford Recruiting NCT01793168
49 Collection of Human Samples to Study Hairy Cell and Other Leukemias and to Develop Recombinant Immunotoxins for Cancer Treatment Recruiting NCT01087333
50 Erythropoietin Role in Acute Kidney Injury (EAKI): a Pragmatic Clinical Trial Recruiting NCT03401710 Erythropoietin

Search NIH Clinical Center for Hemolytic-Uremic Syndrome

Cochrane evidence based reviews: hemolytic-uremic syndrome

Genetic Tests for Hemolytic-Uremic Syndrome

Genetic tests related to Hemolytic-Uremic Syndrome:

# Genetic test Affiliating Genes
1 Hemolytic-Uremic Syndrome 29

Anatomical Context for Hemolytic-Uremic Syndrome

MalaCards organs/tissues related to Hemolytic-Uremic Syndrome:

41
Kidney, Endothelial, Bone, Colon, Bone Marrow, Liver, Heart

Publications for Hemolytic-Uremic Syndrome

Articles related to Hemolytic-Uremic Syndrome:

(show top 50) (show all 5569)
# Title Authors PMID Year
1
Rifaximin does not induce toxin production or phage-mediated lysis of Shiga toxin-producing Escherichia coli. 9 38
17526759 2007
2
Determination of complement factor H functional polymorphisms (V62I, Y402H, and E936D) using sequence-specific primer PCR and restriction fragment length polymorphisms. 9 38
17022693 2006
3
Is factor V Leiden a risk factor for thrombotic microangiopathies without severe ADAMTS 13 deficiency? 9 38
16411392 2005
4
Shear stress and von Willebrand factor in health and disease. 9 38
14631548 2003
5
Assays of von Willebrand factor-cleaving protease: a test for diagnosis of familial and acquired thrombotic thrombocytopenic purpura. 9 38
11992240 2002
6
Problems with platelet counting in thrombocytopenia. A rapid manual method to measure low platelet counts. 9 38
11446657 2001
7
Hemolytic-uremic syndrome and complement factor H deficiency: clinical aspects. 9 38
11446651 2001
8
Function of von Willebrand factor in children with diarrhea-associated hemolytic-uremic syndrome (D+ HUS). 9 38
11446662 2001
9
Recurrent and de novo renal disease after kidney transplantation with or without cyclosporine A. 9 38
2024653 1991
10
The role of von Willebrand factor (vWF) in thrombotic thrombocytopenic purpura (TTP) and the hemolytic-uremic syndrome (HUS). 9 38
2191307 1990
11
Virulence genes in Escherichia coli isolates from commercialized saltwater mussels Mytella guyanensis (Lamarck, 1819). 38
30379238 2019
12
QseC Signaling in the Outbreak O104:H4 Escherichia coli Strain Combines Multiple Factors during Infection. 38
31235511 2019
13
Rab7b participation on the TLR4 (Toll-like receptor) endocytic pathway in Shiga toxin-associated Hemolytic Uremic Syndrome (HUS). 38
31153054 2019
14
Moxetumomab pasudotox-tdfk for relapsed/refractory hairy cell leukemia: a review of clinical considerations. 38
31134324 2019
15
Transcription of the subtilase cytotoxin gene subAB1 in Shiga toxin-producing Escherichia coli is dependent on hfq and hns. 38
31375495 2019
16
Development of a Gold Nanoparticle Vaccine against Enterohemorrhagic Escherichia coli O157:H7. 38
31409688 2019
17
Angiopoietin-Tie signaling in kidney diseases: an updated review. 38
31380564 2019
18
Complement deficiencies and dysregulation: Pathophysiological consequences, modern analysis, and clinical management. 38
31421540 2019
19
Extra-renal manifestations of atypical hemolytic uremic syndrome. 38
30109445 2019
20
Renal Transplantation in Patients With Atypical Hemolytic Uremic Syndrome: A Single Center Experience. 38
31400975 2019
21
Reduced membrane attack complex formation in umbilical cord blood during Eculizumab treatment of the mother: a case report. 38
31390992 2019
22
Placental sFLT1 is associated with complement activation and syncytiotrophoblast damage in preeclampsia. 38
31291799 2019
23
The role of properdin in complement-mediated renal diseases: a new player in complement-inhibiting therapy? 38
30141176 2019
24
Synergistic effects of ADAMTS13 deficiency and complement activation in pathogenesis of thrombotic microangiopathy. 38
31409673 2019
25
Moxetumomab pasudotox for the treatment of relapsed and/or refractory hairy cell leukemia. 38
31298972 2019
26
Defects in complement and "secondary" hemolytic uremic syndrome. 38
31331470 2019
27
Hemolytic uremic syndrome in a developing country: Consensus guidelines. 38
30989342 2019
28
Whole genome-based public health surveillance of less common STEC serovars and untypable strains identifies four novel O genotypes. 38
31366691 2019
29
Pharmacology, Pharmacokinetics and Pharmacodynamics of Eculizumab, and Possibilities for an Individualized Approach to Eculizumab. 38
30758736 2019
30
Use of complement monoclonal antibody eculizumab in Shiga toxin producing Escherichia coli associated hemolytic uremic syndrome: A review of current evidence. 38
31286658 2019
31
Adults with septic shock and extreme hyperferritinemia exhibit pathogenic immune variation. 38
29977033 2019
32
Eculizumab for atypical hemolytic-uremic syndrome. How long should we maintain it? 38
30798995 2019
33
Correction to: Clinical characteristics and genetic backgrounds of Japanese patients with atypical hemolytic uremic syndrome. 38
31016432 2019
34
Whole-exome sequencing detects mutations in pediatric patients with atypical hemolytic uremic syndrome in Taiwan. 38
30905589 2019
35
Atypical hemolytic uremic syndrome in a patient with protein-losing enteropathy. 38
31364428 2019
36
A Multimodality Approach to Assessing Factor I Genetic Variants in Atypical Hemolytic Uremic Syndrome. 38
31312772 2019
37
Terminal complement effectors in atypical hemolytic uremic syndrome: C5a, C5b-9, or a bit of both? 38
31229026 2019
38
Anti-Factor H Antibody Reactivity in Young Adults Vaccinated with a Meningococcal Serogroup B Vaccine Containing Factor H Binding Protein. 38
31270173 2019
39
Differential contribution of C5aR and C5b-9 pathways to renal thrombic microangiopathy and macrovascular thrombosis in mice carrying an atypical hemolytic syndrome-related factor H mutation. 38
30910380 2019
40
An Ex Vivo Test of Complement Activation on Endothelium for Individualized Eculizumab Therapy in Hemolytic Uremic Syndrome. 38
30851964 2019
41
A review of thrombotic microangiopathies in multiple myeloma. 38
31404728 2019
42
Shiga Toxin Producing Escherichia coli-Hemolytic Uremic Syndrome Mimicking Acute Severe Ulcerative Colitis in a Child With Bloody Diarrhea. 38
30934058 2019
43
Norovirus: a novel etiologic agent in hemolytic uremic syndrome in an infant. 38
31277594 2019
44
Gastrointestinal infection-related disseminated intravascular coagulation mimicking Shiga toxin-mediated hemolytic uremic syndrome - implications of classical clinical indexes in making the diagnosis: A case report and literature review. 38
31367625 2019
45
Prodromal Phase of Hemolytic Uremic Syndrome Related to Shiga Toxin-Producing Escherichia coli: The Wasted Time. 38
31290797 2019
46
Hemolytic uremic syndrome and encephalopathy from Shiga toxin-producing Escherichia coli. 38
31332051 2019
47
Escherichia coli-associated hemolytic uremic syndrome and severe chronic hepatocellular cholestasis: complication or side effect of eculizumab? 38
30963282 2019
48
Dynamic Gene Network Analysis of Caco-2 Cell Response to Shiga Toxin-Producing Escherichia coli-Associated Hemolytic-Uremic Syndrome. 38
31288487 2019
49
SMRT sequencing reveals differential patterns of methylation in two O111:H- STEC isolates from a hemolytic uremic syndrome outbreak in Australia. 38
31263188 2019
50
Acute thrombotic microangiopathic kidney injury due to Echis coloratus envenomation. 38
31218895 2019

Variations for Hemolytic-Uremic Syndrome

ClinVar genetic disease variations for Hemolytic-Uremic Syndrome:

6
# Gene Variation Type Significance SNP ID GRCh37 Pos GRCh38 Pos
1 DGKE NM_003647.3(DGKE): c.888+40A> G single nucleotide variant Pathogenic rs1555599211 17:54925466-54925466 17:56848105-56848105

Expression for Hemolytic-Uremic Syndrome

Search GEO for disease gene expression data for Hemolytic-Uremic Syndrome.

Pathways for Hemolytic-Uremic Syndrome

Pathways related to Hemolytic-Uremic Syndrome according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.61 VWF HP CFI CFHR3 CFH CFB
2
Show member pathways
11.79 CFI CFHR3 CFH CFB CD46 C3
3
Show member pathways
11.78 VWF THBD F3
4
Show member pathways
11.65 CFH CFB CD46 C3
5
Show member pathways
11.56 CFI CFHR3 CFH CFB CD46 C3
6 11.54 CFI CFH CFB C3
7 11.37 VWF THBD F3 CFI CFH CFB

GO Terms for Hemolytic-Uremic Syndrome

Cellular components related to Hemolytic-Uremic Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.9 VWF HP F3 CFI CFHR5 CFHR1
2 extracellular exosome GO:0070062 9.86 VWF HP CFI CFHR3 CFH CFB
3 cell surface GO:0009986 9.72 THBD F3 CD46 CD40LG ADAMTS13
4 extracellular space GO:0005615 9.7 THBD HP F3 CFI CFHR3 CFHR1
5 blood microparticle GO:0072562 9.1 HP CFHR3 CFHR1 CFH CFB C3

Biological processes related to Hemolytic-Uremic Syndrome according to GeneCards Suite gene sharing:

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 innate immune response GO:0045087 9.8 CFI CFH CFB CD46 C3
2 proteolysis GO:0006508 9.76 HP CFI CFB ADAMTS13
3 complement activation, classical pathway GO:0006958 9.67 CFI CD46 C3
4 blood coagulation GO:0007596 9.67 VWF THBD F3 ADAMTS13
5 platelet activation GO:0030168 9.62 VWF DGKE CD40LG ADAMTS13
6 complement activation GO:0006956 9.56 CFHR1 CFH CFB C3
7 positive regulation of interleukin-10 production GO:0032733 9.48 CD46 CD40LG
8 hemostasis GO:0007599 9.46 VWF THBD F3 ADAMTS13
9 positive regulation of cytolysis GO:0045919 9.43 CFHR5 CFHR1
10 complement activation, alternative pathway GO:0006957 9.26 CFHR5 CFH CFB C3
11 regulation of complement activation GO:0030449 9.17 CFI CFHR5 CFHR1 CFH CFB CD46

Molecular functions related to Hemolytic-Uremic Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 9.77 VWF THBD HP F3 CFI CFHR5
2 serine-type endopeptidase activity GO:0004252 8.92 HP F3 CFI CFB

Sources for Hemolytic-Uremic Syndrome

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